Purpura fulminans: a dermatological emergency revisited

Purpura fulminans (PF) is a dermatological emergency with a high mortality rate, characterized by extensive skin necrosis due to microvascular thrombosis. As a manifestation of systemic coagulopathy, PF is frequently associated with disseminated intravascular coagulation (DIC) and sepsis. Dermatologists play a crucial role in its early recognition and multidisciplinary management. This review aims to provide a comprehensive overview of PF, including its clinical presentation, classification, underlying pathophysiology, diagnosis and current management strategies. A systematic analysis of existing literature relating to PF was conducted, focusing on its clinical features, histopathology, laboratory investigations and treatment approaches. PF presents with painful, well-demarcated purpuric lesions that evolve into necrotic eschars. The disease is categorized into neonatal, postinfectious and acute infectious subtypes, with acute infectious PF being the most common. PF pathophysiology involves acquired or congenital deficiencies in proteins C and S, leading to unregulated thrombosis and vascular damage. Diagnosis requires a combination of clinical assessment, coagulation studies and histopathology. Management includes broad-spectrum antibiotics, coagulation support, protein C replacement (in neonatal patients), therapeutic plasma exchange and skin-directed treatments. Surgical intervention is often necessary for extensive necrosis. Given its life-threatening nature, PF demands heightened awareness in the dermatology community. Early diagnosis and prompt intervention can greatly improve outcomes. Strengthening interdisciplinary collaboration between dermatologists, intensivists and haematologists is essential for optimizing patient care and reducing mortality associated with PF.